Severe factor X deficiency in a twin pregnancy

Beksaç, Mehmet Sinan; Atak, Zeliha; Özlü, Tülay

doi:10.1007/s00404-009-1090-4

Cited by 12 publications

(27 citation statements)

References 5 publications

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“…As mentioned earlier, four pregnancies resulted in a miscarriage (13%), three in the first trimester and one was a late miscarriage at 21 weeks . Only one case had prophylactic treatment (twice‐weekly pdFX) and miscarried at 6 weeks.…”

Section: Antenatal Complicationsmentioning

confidence: 66%

“…Two cases did not receive prophylaxis; including one miscarriage at 21 weeks and one first trimester miscarriage (gestational age not specified). In the fourth case, prophylaxis administration was not mentioned and it was a first trimester miscarriage (gestational age not specified) …”

Section: Antenatal Complicationsmentioning

confidence: 95%

“…Gestational age at delivery for the preterm live births varied from 25 to 37 weeks. Mode of delivery was caesarean section in three; a twin pregnancy at 34 weeks, a case of pregnancy‐induced hypertension at 34 weeks and one for preterm labour at 33 weeks . Four were vaginal deliveries; in one case, the gestational age was reported as late second trimester resulting in neonatal death (hypoplastic heart and bleeding diathesis in infant), and in the other, three gestational age reported as 25, 32 and 34 weeks, respectively, the first also resulted in a neonatal death (no data on status of neonate) .…”

Section: Pregnancymentioning

confidence: 99%

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Congenital Factor X deficiency in women: A systematic review of the literature

Spiliopoulos

Kadir

2019

Haemophilia

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Factor X deficiency (FXD) is a rare autosomal recessive bleeding disorder with a variable phenotypic severity. In women, heavy menstrual bleeding (HMB), recurrent ovulation bleeding with haemoperitoneum and bleeding complications in pregnancy such as retroplacental haematoma and postpartum haemorrhage have been reported. The aim of this review was to examine gynaecological problems and obstetric complications in women with congenital FXD. A total number of 49 relevant articles were identified, including 332 women, dating from 1960 to 2018. Heavy menstrual bleeding was reported in 72/284 (25%) women in total, 14/30 (47%) in case reports and 58/254 (23%) in 11 case series, 64% and 10% required blood products and blood transfusion, respectively. Haemoperitoneum from ovulation bleeding or ruptured haemorrhagic ovarian cyst requiring blood transfusion occurred in 8/322 (2.4%) women, six required surgical intervention, including oophorectomy in two. 31 pregnancies were reported in 19 women. There were four miscarriages (including a late miscarriage at 21 weeks). There was a high rate of preterm birth and neonatal death occurring in eight (30%) and three (11%) of pregnancies reaching viability stage. Postpartum haemorrhage (PPH) occurred in six (22%) of deliveries, one requiring hysterectomy. In conclusion, women with FXD are at an increased risk of heavy bleeding during menstruation and ovulation as well as adverse pregnancy outcome and postpartum haemorrhage. Collaboration in a multidisciplinary team including an obstetrician/gynaecologist, a perinatologist and a haematologist is necessary for the prevention and management of these complications.

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Section: Antenatal Complicationsmentioning

confidence: 66%

Section: Antenatal Complicationsmentioning

confidence: 95%

Section: Pregnancymentioning

confidence: 99%

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Congenital Factor X deficiency in women: A systematic review of the literature

Spiliopoulos

Kadir

2019

Haemophilia

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“…The FX gene is 22 kb long and is located at 13q34-ter, 2.8 kb downstream of the F7 gene. The coding sequence is homologous to other vitamin K-dependent proteins such as FVII, FIX, and Protein C, and is divided into eight exons, each encoding for a particular domain within the protein (3,4). FX is a vitamin K-dependent liver-produced serine protease, which serves an important role in the normal coagulation mechanism.…”

Section: Introductionmentioning

confidence: 99%

“…In addition, the prevalence of the homozygous form is one in 500/000 -1 000/ 000 of the general population. According to the levels of patients' plasma FX, the clinical manifestations of FX deficiency are varying from severe (< 1%) to mild (6% -10%) (4,5). Patients with moderate-severe deficiency (factor X level below 1%) may have symptoms similar to those of hemophilia A and B, including haemarthrosis, intracranial hemorrhage, and gastrointestinal (GI) bleeding (5).…”

Section: Introductionmentioning

confidence: 99%

Subarachnoid Hemorrhage in Congenital Factor X Deficiency: A Case Study and Literature Review

Mohammadi

Torab

Aghakhani

et al. 2016

Iran Red Crescent Med J

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Background: Inborn factor X deficiency (FXD) is a very rare (1: 500,000) hereditary coagulation disorder, which is characterized by clinical manifestations including hematoma, epistaxis, menorrhagia, ecchymosis, and central nervous system (CNS) or gastrointestinal (GI) bleeding (depending on the zygosity). In homozygote patients, the risk of spontaneous intracranial hemorrhage (ICH) is high. Objectives: The aim of this investigation was to study and long-term follow-up of the patients with FXD and ICH. In addition, we investigated their frequent bleeding symptoms throughout their life and the results were compared with results of other studies. Patients and Methods: This study investigated 2 cases with spontaneous intracranial hemorrhage in patients with severe congenital (factor X) FX deficiency including a 3-year-old boy and a 1-month-old female neonate. The world literature was explored through the PubMed Medline and Scopus using appropriate and pertinent key words. Results: The Patients referred to the hematology department due to the neurological complications such as vomiting, unconsciousness, prolonged nasal bleeding for recent 12 hours. They had no familial history of spontaneous CNS bleeding. The blood coagulation test analysis indicated a prolonged activated partial thromboplastin time (APTT) and also revealed a prolonged prothrombin time (PT) and the low levels of coagulation factor X implicating severe congenital FX deficiency. They followed up by our hematologists to prevent intracranial hemorrhage. Discussions: As one ICH patient whose PT and aPTT suggest a coagulation disorder secondary to vitamin K deficiency or coagulation factor deficiency, unresponsiveness to vitamin K therapy should be useful to take FX deficiency into consideration.

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Factor X Deficiency

Brown

Douglas

2018

Consults in Obstetric Anesthesiology

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Severe factor X deficiency in a twin pregnancy

Cited by 12 publications

References 5 publications

Congenital Factor X deficiency in women: A systematic review of the literature

Congenital Factor X deficiency in women: A systematic review of the literature

Subarachnoid Hemorrhage in Congenital Factor X Deficiency: A Case Study and Literature Review

Factor X Deficiency

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